January 5, 2010

PFAPA update

In the past, I've shared about Ivy's periodic fever syndrome. It has been quite a while since I've given an update.
During the spring we took Ivy to an infectious disease doctor, Dr Russ Osgathorpe. He is a wonderful pediatric specialist. When we went, Ivy had just finished an episode two days before. When he looked at her throat he saw the typical ulcers a child with PFAPA gets. He said that while we were talking about her, he was questioning if PFAPA was the right diagnosis. But after looking at her throat, he was 95% certain that that is what it is. He convinced us to try the prednisone treatment and said if she responded to the prednisone then he would be 100% confident in the diagnosis.
Three weeks later we got to give it a try. When she spikes to at least 101 degrees, we are to give her 1 tsp and then again 12 hours later. The several times we have given it to her, the fever breaks within the hour and doesn't return. Her fevers are less frequent now and with the prednisone, they never get above 101.
We are thrilled with our results.
During the Christmas break, she had an episode. We knew two days before the fever hit that she was starting. As you can see in the pictures below, her eyes start to get a sad looking and she drinks a ton of milk. This time, she enjoyed a root beer float.






Our next step is to get her tonsils and adenoids removed. There is an 80% chance of a cure- which seems like pretty good odds- especially now that we are comfortable with her diagnosis.
If you know of a child that has recurrent fevers that start to fit into a schedule of every 3-4 weeks, suggest they talk to their doctor about PFAPA. The doctor will run a few tests, to rule out anything else. The first test will be blood work measuring their sed rate during a fever and when they aren't fevering. They'll also run a urinalysis and culture to rule out a urinary tract infection. If their tonsils are affected, they'll run a strep test.

63 comments:

Emile said...

I'm pretty certain that my 3 yr old daughter has this. She's had the fevers for 2 yrs already & we are finally getting close to having some answers (I think). Her regular ped. just kept blowing us off until I finally demanded to see a specialist. We're taking her to an immunologist now and are in the process of awaiting lab results to rule out other possibilities. She has the mouth ulcers as well & now that I know about PFAPA and have been reading up on it - I feel it describes her syndrome well. How is your daughter now? Did you do the tonsillectomy?

Anonymous said...

My son has had pfapa for the past three years. We have seen all kinds of specialist. All but one doctor told us to wait it out, and avoid getting his tonsils out. "why bring mortality into a benign condition" was what I was told by one and I have never been able to get that out of my mind. He has the syndrome as I type, perhaps this will be the last month...

amy said...

I so wish I had both of your email addresses to respond.

Ivy had her tonsils out and we went 5 months without a fever. We just had her first fever last week since the tonsillectomy. It is amazing how much she has changed since she had her tonsils out. She has more energy and she's enjoying everything more than she ever did before.

Was it a cure? It doesn't appear to be, but we've only had 1 fever in 5 months and it only lasted about 36 hours.

For us, the decision came down to was the risk of a fairly simple procedure worth it for the chance that she would not have to have a fever every month for the next 3-10 years. We opted for the risk, the procedure went smoothly and we're still glad we did it.

Sure, it is only an 80% chance of a cure. But depending on the source, a child won't grow out of PFAPA until they are 6 (the youngest I've heard) or going through puberty. If she outgrows it when she's 13, that is a really long time to have to deal with feeling like crap every 3 weeks for a week.

But, bottom line is that you have to do what feels right for you and your child.

Patrick said...

My son had PFAPA. He started getting fevers at age 3. Before being diagnosed, it drove us crazy and made us so sad. He would get clockwork fevers of 104+, and every time we took him to the pedi we were told it was either a) a virus or b) he was put on antibiotics. Of course, under either diagnosis the symptoms went away in 5 days. Two things made us suspicious. First, his brother never got sick, which ruled out a virus; and secondly, he got a fever more than once while on antibiotics. We took him to a natural healer who tried everything (candida cleanse, no milk). He missed so much school and activities...

The I came across some information on PFAPA and thought that I had written the posts. They described my kid to a T. We brought it to the attention of our pedi, who researched it, looked back on his medical records, and agreed that this was what he had. She referred us to a specialist.

As parents, we tried to stay as natural as possible, and don't believe in surgery unless absolutely necessary. In this case we decided that our son had been through enough (he was now 10) and there was a chance it could last til he was 14. We had his tonsils out, and the change has been so incredible. He has had only one fever in almost 18 months, and we are sure he is out of the woods.

The evidence for surgery is much better than some people are aware. There was a randomized study done in Italy. 39 kids with PFAPA, surgery on 19, placebo for 20. They were followed for 18 months. Of the 13 kids having no further episodes, 12 had had surgery. Of the remaining kids who had surgery, the average number of episodes in the 18-month followup was 0.7, vs 8.1 for the placebo group. The real convincing point for us was our surgeon told us he had done over 100 of these surgeries for PFAPA (including his own daugther), and all but one had completely resolved their fevers.

A bit long winded - sorry. But I believe this is a lot more common than people know. Here is a link to the study:

http://www.medscape.com/viewarticle/710276

Best of luck

amy said...

Patrick- thank you so much for your comment. I am so glad you have had success with the tonsillectomy. It is surprising how well it works. Ivy has only had 1 fever in 6 months and her energy is incredible. She is happier and more bubbly. She's known so much pain in her short life that to have the pain completely gone must just feel amazing!

Anonymous said...

I'm pretty sure my 21 month old son has this. He has been having fevers every 3 or 4 weeks like clockwork. I don't know if he has any of the other symptoms though. His only other symptom is vomiting. Have any of your children had vomiting when they have the fever? Thanks so much for writing about this subject.

Misty said...

Our middle daughter is being checked for this. She just turned 8 in July. She has had the fevers since she was a baby. I've only seen the mouth ulcers once. She is also being tested for TRAPS. Surgery is out for us because she also has Ehlers Danlos - Hypermobility Type. I literally laughed out loud when the peds tried to tell me that yet again she had Hand, Foot and Mouth Disease (she gets a rash on her palms and soles) I whipped out my journal of fever dates and explained how we planned our vacations around them and suddenly it wasn't Hand Foot and Mouth, haha. Her fevers are farther apart and not so high (we were at 106 in the beginning, now they don't go any farther than 102.5). We had no episodes for 5 months and just had one 3 weeks ago, right before she also got strep. I'm pretty confident hers is starting to come to the end. She's a tough little cookie. Missed a ton of school but is an A student. I have no idea how she does it. For those of you than do not choose surgery (or can't like us) there is hope that it gets better. It was gradual for us but it's progress. Good luck to you and your little ones, with 7 years in I know the struggle all too well.

Laura Funke said...

Only tonight did our pediatrician call and told us about PFAPA. Our son is 3.5 years now, but has been getting fevers and mouth blisters since he was a little baby, probably 6 months. The episodes always last 7 days, although they are not nearly as frequent as some of the other posts I have read here. We probably go 3-4 months between episodes. He is having an episode right now, it is really bad, probably one of the worst he's had. He screams and kicks and I try to hold him and we cry together. Our peditrician last night finally said that he wanted to talk to an infectious disease specialist and within 24 hours he had called back and said, I think your son has PFAPA. It's kind of hard to process right now, I am reading a lot on the web. But I just don't know what to do from here.

amy said...

Hi Laura-

We've tried several different treatments. The first thing we tried was tagament. Then we tried clariton. After that we tried Emergen-C. They all bought us several months without fevers. For you, it could buy you more than a few months - if yours are only every 4 months.

good luck with this and I hope things go better.

Mandy said...

My daughter's ENT doctor said that she may have PFAPA. I immediately went online to find out what it was. The sypmtoms match my daughters to a tee (fever every 6 weeks, headaches, fatigue, etc.) I was so happy to know that my "mother's intuition" was right again (after hearing from her pediatrician only Friday that it's "normal" for my daughter to be sick so often). Obviously I didn't believe that!! I am very hopeful that she will have her tonsils out and that she will not be suffering so much. Thank you for your site and sharing your child's struggles with the illness as well because it gives me a lot of hope for her future!

Mandy said...

My daughter's ENT doctor just told me today that my daughter may have PFAPA. I've been begging her pediatricians to acknowledge what I had been seeing the past few years (fevers every 6-8 weeks like clockwork, headaches, fatigue, etc.). After hearing "it's normal" I was starting to doubt that my concerns were valid. Thank GOD for mother's intuition! I decided to give her ENT doctor one more chance and he finally diagnosed her with PFAPA. After reading the research and what other parents have experienced, I know that this diagnosis is exactly what she has. Although I am concerned about her having surgery at age 8, I am confident that she will feel a lot better and I can finally get some piece of mind. I feel better knowing that my daughter is going to be much better once she has the surgery.

Salana said...

My son is 7 and was diagnosised with PFAPA only 1 1/2 years ago. He was so sick at that point we ended up at Children's Hopital in Dallas with an ID doctor. He was 100% sure about what was going on with him. We had fever every 3 weeks and ulcers in mouth since he was 1 year old. He was going through and eposide when we were diagn. He had seen ENT's and they had orderd MRI cat scan thinking that he had a sinus infection that would not clear up. They then put him on 28 days of antibiotic at 500mg a day. He developed C-Diff along with the fworst episode of PFAPA we could have ever had. He missed 4 weeks straight of school with fever and not able to keep anything he ate in his body because he had very little good bacteria left in his system because of the antiboitic. We had his tonsils taken out that summer, and what a huge difference it made. He was only sick a couple of times with PFAPA. This year he has just gone into the 3 week cycle now. We do the predisone and tagmet. IT really makes ahuge difference with him. To any parent out there if your not happy with what the Drs tell keep going until you get an answer.

the quirky one in the family said...

I just found your post tonight while I was looking up more info on PFAPA, thanks for posting Ivy's trip down the PFAPA road. DR. Osguthorpe diagnosed my son last year with it and we had his tonsils out in November, but he recently had another 7 day episode. We did get two months free from fevers, but his come with seizures and 104-106 temps. I haven't read anywhere of any statistics of recurrence post surgery, but would love to hear from you if you had. My little guys is turning 2 this week, so we are looking at a long road left in his PFAPA trip if his fevers are still going to come every month. I would love to hear if you had heard anything about post surgery PFAPA kids. Thanks so much!

Patrick said...

Quirky one:

Patrick here - I posted above. I just saw your post and thought that the link I posted would help. However I tried following my link and got prompted for a username and PW. Sorry about that - everyone.

Google "PFAPA" and "medscape" and then choose the link that starts "Surgery for PFAPA Syndrome?..." This will allow you to view the article about the surgery study without a username and PW.

In case you can't get to it, here are the important parts:

"The authors enrolled and randomly assigned 39 children. Nineteen patients underwent surgery. Before surgery, the groups were similar when compared by episodes of PFAPA in the prior year (mean, approximately 8 episodes), duration of episodes, and maximum fever during episodes.

The authors had complete retention of study participants through the follow-up period. Of the 13 children who experienced no further episodes of PFAPA, 12 were in the surgical group. By comparison, 63% of the surgical group vs only 5% of the control group had no further episodes of PFAPA in the 18-month follow-up period. Some patients who underwent surgery did experience a PFAPA episode after surgery, but the mean number of episodes after enrollment (and surgery for the surgical group) was much lower in the surgical group at a mean of 0.7 episodes per child vs 8.1 episodes per child over the 18-month follow-up period. None of the surgical patients experienced more than 3 episodes of recurrence after adenotonsillectomy.

The authors concluded that adenotonsillectomy is a highly effective therapeutic option for children with PFAPA syndrome."


It is sooooooo painful and stressful to be free of the fevers for awhile and then one hits. But don't worry - based on the study statistics your son is so close to being out of the woods. If it helps, my son had surgery in February a couple of years back. He went until June before another episode. That was his last one - ever. So hang in there.

The only other observation I had that might help is that after surgery my son gained weight. We eat extremely well and he is active. I talked to a couple of old-timers who had kids/were kids back when tonsils came out regularly. Without any prompting, they all mentioned short-term weight gain. My son now looks fitter than I could ever have hoped.

Patrick

Anonymous said...

My four year AND my 17 month old have been diagnosed with PFAPA. They have both had all the symptoms including 106 degree fevers every 28 days since May of 2010. We are desperate and trying the tonsillectomy on our 4 year old on Feb 1. We are so scared but at our wits end and praying this works. Thanks so much for all your posts...it gives me great hope and makes me feel better about our choice to try this. I will keep you all posted!

Anonymous said...

Wow - I have a 4 year old little girl who has been having "mystery fevers" since she was about 6mo old. We did blood work when she was about 1yr (but I'm not sure what they were looking for). We were military & when we changed dr's, we finally got tubes in her ears (because of ear infections). I asked about doing some more tests (she was 2 then) They said keep a journal - um, hello you have her med records right there!

Anyway, after being told all the same stuff, must be a virus (but my older daughter never catches it), they give us anti-biotics & move us along. She just went thru 6 days of 103.3 & they tested her for flu, for strep, checked her ears (tubes out - no ear infection) I am at wits end & it dosn't help that I have a friend who's little girl went thru fevers & infections to be diagnosed with cancer. So I am sick with worry waiting for a fever to come on (about 1 or 2x a month) & sick with worry when they come. I can't go on a weekend trip with my husband because I am so worried about her getting sick while I'm gone. My work is fed up with me missing time. I feel like noone believes me this little girl is sick! I started to research & found your blog & started to cry. I am printing this off to show her Dr. Of course now that we are no longer military all testing we have to pay for, but I don't care I just want her to feel better!

What should I be asking the Dr to look for? How do I make them listen to me & stop dismissing me?

Vanessa
imagine.that@streetstylephotograhpy.net

sherri said...

My 7 year old daughter struggled for years ( from age 1 1/2 - 5 1/2) with PFAPA. Every time we went to the Dr. they checked her for Strep, which was always negative & sent us home with a Viral diagnosis. No one ever mentioned the possibility of this to me until she was 5. She had fevers every other week for 4 years & we were desperate since she was about to start school & I knew she would miss so much. I started keeping a journal of fevers & dates. Our pediatrician took one look at the journal & said I think she has PFAPA. We were sent to Vanderbilt Children's Hospital to Infectious Disease & they took my journal & put every date into their records. Long story short, we tried steroids which made fevers more frequent but only last 3 days instead of 7. Then we tried Tagamet & Cimetidine which only helped a little. Finally, after looking at blogs about it, I suggested to our pediatrician we have tonsils & adenoids out. He & the Dr.'s at Vanderbilt weren't totally for it- they said there was no evidence to prove it cured it. I called her ENT & he had done the surgery on 5 kids & said none of them had fevers anymore. So, we went in to surgery with a 105 fever in the middle of an episode & she came out of recovery with NO Fever! It has been 1 1/2 years and no fevers. She still occasionally has joint pain, headaches, and fatigue, but never a fever. We are so happy we did it! The Dr.'s at Vanderbilt did mention that participants in their study sometimes had fevers return during puberty & pregnancy for girls. We are keeping our fingers crossed!

THE WHITTINGTONS said...

I just found your blog while researching PFAPA. My 3 year old son, in addition to his other medical issues, has all the symptoms of PFAPA (fever every 3 weeks, throat ulcers but negative strep tests, etc). We are in the midst of tracking these episodes and doing blood and urine tests. It will be nice to at least have an answer to the frequent fevers. I feel so sad for my little guy.

lisa said...

I was so glad to find this blog! My little 2 1/2 year old daughter was just diagnosed with PFAPA. She's been having recurring fevers since March of 2010 and all I got from her pediatrician was "Oh it's just a virus." Really? Every month like clock work? I don't think so. We were referred to an Infectious Disease specialist who did extensive blood work to rule out major issues. On the follow-up visit he gave a prescription for Prednisone...if she responds to the dose during the next fever episode he's almost positive of PFAPA. She took her first dose 3 days ago and vomited almost immediately after, so I'm sure nothing absorbed in her system. But by the end of the night the fever was down and she was more like herself. No fever for the next two days and them BAM! Today it spikes to 103 out of the blue. Hopefully another dose of Prednisone will work.
Thank you all so much for sharing your stories! It helps to know that there are other parents/children enduring through this same insane thing!

amy said...

My 3.5 year old daughter was told this morning she may have PFAPA. I found this blog and so glad I did. Though my daughter has only had two episodes of fever (19 days apart) of 102.9 (or lower) the doctors are taking it very seriously. She lost 2lbs during her last episode (fevered for 8 days) and she only weighs 26 lbs now. She is scheduled to see a hematologist for anemia and the doctor wants me to call him in the morning with her temperature. I'm just really glad that I am getting somewhere and they are taking this very serious. I couldn't imagine having to wait years to have a diagnosis. We shall see what happens. Her first episode resulted in Negative tests for Mono, Strep, Ear Infection, and anything else they can rule out with a CBC. They took four tubes of blood from her. I have no idea what else they tested for, but all tests were negative except for she is a bit anemic. Besides the anemia the only other diagnisis I have is PFAPA. Thanks for the blog and information.

liat said...

Hi. My 4 year old son was diagnosed..it is a relief to have some answers. I am looking for a specialist in the NYC area to help with a course of treatment. Can anyone out there recommend someone? Thanks in advance.

liat said...

Hi. My 4 year old son was just diagnosed with PFAPA. What a relief. I am looking for a specialist in the NYC area that can help with a treatment course.. Can anyone recommend someone? Thanks in advance.

Anonymous said...

How many fever episodes did your child have before they decided it was PFAPA? I went to the doctor today with my son who has had a fever for 8 days now and he said if it happens next month he is going to send us to infectious disease. He had a fever most of the month of January but none in Feb.

amy said...

The first month Ivy had a fever for most of the month. Then we skipped a month and then it was back the third month for about two weeks.

It took about 6 months before they determined that it "might be" PFAPA but that was after they had ruled out so many other illnesses.

But we weren't confident about the diagnosis until we tried the prednisone and it responded exactly the way the doctors said it would for PFAPA.

lisa said...

Anonymous,
My daughter had 6 episodes spread 9 months apart before we went to the specialist for the initial visit. 3 months later at the next follow up (and 3 more episodes later) he diagnosed her with PFAPA. Her fevers only lasted for about 4-5 days though.
Wish you all the best!

Christen said...

Thank you for the information Amy! Is it hard living with PFAPA? Is it difficult for you little girl? Hopefully Will's fever will be gone by Monday otherwise the doctor is going to call infectious desease.

katie said...

hi, there--

just this morning my pediatrician brought up PFAPA. after reading all of these comments, i am feeling grateful to have the doctor that we do. my daughter is 23 months and has had fevers in the 103-105 range every month, almost to the day, in 2011 and previous fevers every 2 months before that in 2010. the more i read, the more convinced i am that she has PFAPA. realizing how debilitating this could be once she reaches school age and would have to miss a week every month with a non-contagious syndrome that presents with a fever really started to make me realize what struggles that all of you go through.

we are just starting on a road to a definitive PFAPA diagnosis. i appreciated being able to read this blog. thank you.

Jennifer said...

it's great to see stories from others going thru the same thing. My son Brennon is 6 years old and has been having episodes since he was 2. We finally have a diagnosis of PFAPA that we received in aug 2010 after 4 years of antibiotics, shots, blood tests and multiple multiple doctors visits. We ahd a nice brek of 5 months this year but as of march we have had 3 episodes 2 weeks apart. Just today our pedi suggested sending him to an ENT docotor to discuss tonsil removal. we re hopeful it will stop the episodes compltely. Though we do use the predisone and it does help to stop his fevers. we call them his magic pills...

Patrick (part 1 of article) said...

Hey everyone:

It is not often we get new news about this issue, but the following was posted in the April 17th Ontarget newletter (you can join by going to http://www.targethealth.com/ontarget.aspx and registering). A potential option to go along with surgery and corticsteroids. The actual proposed treatment is all the way down in the second-to-last paragraph. Pretty good news...

I need to post in two parts

Cause and New Treatment for Common Recurrent Fever in Children Identified

A syndrome called periodic fever associated with aphthous stomatitis, pharyngitis and cervical adenitis - or PFAPA, is characterized by monthly flare-ups of fever, accompanied by sore throat, swollen glands and mouth lesions. Children with PFAPA syndrome experience attacks of fever, each lasting three to six days, usually three to eight weeks apart. Their predictability is so regular that parents have been known to make pediatric appointments a week ahead of when they expect their child to experience a PFAPA episode. Affected children experience their first attack before the age of 5, with fever episodes usually abating in adolescence or young adulthood. While the only remedy for PFAPA, besides corticosteroids, is removal of an affected child's tonsils, which has a good rate of success in eliminating PFAPA syndrome, but is an invasive alternative.

Patrick (part 2 of article) said...

Part 2:

According to a study published in the early online edition of the Proceedings of the National Academy of Sciences (8 April 2011), has identified a promising new treatment in children for the most common periodic fever disease in children. The proposed treatment, which will be validated in a larger study before it is recommended in treating PFAPA syndrome, wards off an inappropriate immune system attack without increasing the frequency of flare-ups, a problem caused by the current standard treatment with corticosteroids.



“Until now, the basis of PFAPA has been a mystery,“ said senior author and NHGRI Scientific Director Daniel Kastner, M.D., Ph.D. “Advances in genomic analysis have allowed us to define a major role for the innate immune system, the body's first line of defense against infection. Targeting a specific product of white blood cells at the first sign of fever appears to abort the attacks.“



The new experimental treatment resulted from the authors using a systems biology approach, which entailed gene and protein expression and cell biology analysis in carefully selected patient and healthy control subjects, to determine the underlying disturbance of the immune system. They analyzed patient blood samples to detect which gene and protein networks are involved in the cell signaling and metabolic pathways activated in the disease. The study evaluated 21 patients with PFAPA syndrome along with an equal number of healthy children and 12 children with a distinctly different set of hereditary fever syndromes. They analyzed gene expression during episodes of fever and intervening periods when the children were well. The analysis revealed gene expression profiles that uniquely identified PFAPA immune response.



During PFAPA flare ups, the authors detected activation of both forms of immune response: 1) the innate first-line-of-defense immunity, and 2) adaptive immunity, which is the body's ability to detect and remember an infection in order to fight it later. This dual response supports the idea that the fevers of PFAPA are an immunologic response to some external stimulus, possibly related to microbial infection.



The authors looked for biological markers that would indicate the onset of a flare-up of fever in children with PFAPA. During PFAPA flare-ups, the authors detected decreased numbers of activated T cells, white blood cells that play a role in the cell's innate immune response. They suspect that these activated T cells migrated to the lymph nodes in the neck, where they accumulate. They also detected over-expression of genes activated in innate immune responses, including interleukin-1, a molecule that is important in triggering fever and inflammation.



From these data, the researchers hypothesized that anakinra, a drug that prevents interleukin-1 from binding to its receptor, could be therapeutic. They administered anakinra by injection to five children on the second day of their PFAPA fevers and all showed a reduction in fever and inflammatory symptoms within hours.



“The anakinra treatment has the potential to restore these children to a mostly symptom-free childhood,“ said Dr. Kastner. “The comprehensive analysis of gene expression during PFAPA attacks would not have been possible without the tools created by the Human Genome Project, and the possibility of an effective treatment is yet another of the genome project's many dividends.“



A larger clinical trial for the use of anakinra in treating this periodic fever syndrome is planned, Dr. Kastner said.

katie said...

to patrick...

wow! so interesting! my cousin is a genetic counselor and i plan on passing on this news, as well as bringing to our next doctor visit (planned with an assumption of a PFAPA episode occurring the first week of may).

it is my little girl's 2nd birthday today, and this is exciting news to have! we have been getting the fevers at the beginning of every month (last one was 2 weeks ago), but she just had another flare up this weekend.

it sounds weird to "hope" for PFAPA, but i sure would like this to be our answer. (as i previously posted, we are in diagnosis mode)

thank you!

Anonymous said...

Get your child's tonsils and adenoids out!

My son was diagnosed w/ PFAPA at age 3. We saw the Infectious Disease specialist at DuPont Hosp. in Wilmington, DE. Also saw same at CHOP (Children's Hosp. of Philadelphia). Were told that he would need to take prednisone, etc. I was not happy, as my son would be on steroids more than once a month. So I only gave him Tylenol, etc. to make him more comfortable.

Long story short: saw ENT at DuPont who told me that it is a fact that removing tonsils and adenoids were the cure.

He also indicated that infectious disease specialists do not wish to recommend the operation, and string you along, medicating the child, not curing the child. This ENT (a God-send!) indicated that he had written an article for JAMA, but they would only publish it in their ENT section, not their Infectious Disease section, so Infectious Disease specialists did not get the chance to read it.

My son had the safe prodecure performed when he was 7. The Doctor indicated that, upon looking at the removed tonsils, they were filled w/ pus. The tonsils are the source of this cyclical infection.

My son is now 10. He has NEVER had an episode since. After languishing w/ the build-up to a fever, the fever itself for 3 days, and the exhaustion from going through a fever every three weeks, for more than 4 years, my son is now a far more energetic boy, who does not fear getting fevers.

A friend of mine has a son who was diagnosed w/ PFAPA recently. He has already seen the specialist at DuPont, and is scheduled for surgery. Wish I had done that much sooner than I did.

Please contact a pediatric ENT, or, if you get no satisfaction, call DuPont Hospital.

amy said...

I completely agree that having their tonsils out is a WONDERFUL option for a cure. It is only a cure for 80% of those kids that suffer from PFAPA but an 80% chance is worth the surgery.

Ivy still has "episodes" that include all of her other symptoms but her fever rarely gets above 100.5 - which as far as I am concerned is a VICTORY. They are no longer regular but they are very obviously PFAPA episodes.

T&A-ectomy is definitely the way to go. I hope everyone falls in that 80% that cures it.

Carol said...

Hello Amy,

I have a 5yearold son that for the last year was bean having fever every month..
So every time that happens I take him to the doctor and to the emergency room..

Las month I saw a doctor that told me he was PFAPA..

For now the doctor sent me to a immunology and Rheumatology..

Please I need more information on what specialist doctor to go to..

Can you help Me please..

Thanks..
please e-mial me at garota_carioca83@hotmail.com

Stacey said...

Can't thank you enough for your blog and the many comments that followed your PFAPA update post. My 3 yo is experiencing her 6th fever in 5 months (every 3 weeks like clockwork). My daughter also had fevers her first year every 6 weeks, but had none her whole 2nd year. The past two have had mouth ulcers with the fever. After 2 rounds of blood work and still no huge answers aside from slight anemia, she's come up with another fever. Glad to know I am not alone and will be making a call on Monday for another visit (I'm on a first name basis now...sigh) and possibly an infectious disease recommendation. Thanks again for the info, the studies and encouragement! This is very possibly what she is having.

Anonymous said...

I started having very high periodic fevers when i was 4 months old, accompanied by swollen lymph nodes and nausea/vomiting. It continued until I hit 6th grade. when I was in 5th grade my pediatrician said that I may have PFAPA. I am now 15 years old and this year my symptoms have come back. every time i get run-down or the flu is going around i show signs of PFAPA instead. your stories have been very helpful, but I have yet to read of a child have symptoms reappear after a sustained amount of time. If you have any info please share, thank you

katie said...

hi, again--

my daughter had her T/A surgery last week. the recovery has been terrible (she's 2, they say it is usually tough for the little ones). once we get past this, then we wait and hope that it cured the PFAPA.

i found your blog at the beginning of our journey. luckily for us, it has been a much shorter one than many of you have had to endure. i just want to say thank you for the blog. it was a real help in those first days of learning about PFAPA. here's to hoping that my daughter is able to put it in her past!

with thanks,
katie

amy said...

Hi Katie-

I hope it works for you. I'm so glad that this post and all of the wonderful comments have helped. I'd love to hear if the T&A worked for her. We'll all keep our fingers crossed.

Robin said...

Thank you so much for posting this. My 4 year old has been spiking 104+ fevers regularly for about 6 months now. Today I took him to the Dr. and looking at his medical history he immediately said he thinks he has Periodic Fever. In googling it when I got home, PFAPA fits him to a T. Right now we're in the process of ruling out everything else (aka about 2 dozen vials of blood drawn for tests, poor little guy). The experience you have shared on your blog, as well as all the comments, have truly given me hope! Thank you so much for sharing Ivy's story!

Lauren L. said...

Hi :] i'm lauren and i was the 101st case of PFAPA in the U.S.
I was only one year old when i first started showing signs of this, and I was diagnosed at age three.
I'm 17 now.
I never had any treatment for it and i never had my tonsils out, either. They told us there was nothing we could do so my mother would just give me ibuprofen for the fevers the day or a couple days they knew i would start.
As I grew up, most all the symptoms have stopped. the only things i still get are little ulcers in my mouth and leg cramps.
but I wouldnt call them 'episodes' anymore. just things that happen :]
PFAPA is a disease where I grew out of. And I've read online, also that you grow out of it too.
I don't even remember me getting fevers and stuff. I'm healthy and everything too :]
I sometimes get sick easily though. I sometimes wonder if that is because of PFAPA bringing my immune system down, but nothing happends where its life threatening. I wish best of luck to your baby girl. she's precious. And i'm sorry she has to go through with this.
But it will get easier over time. :]

Vanessa said...

To Patrick.

My daughter is nearly five and was diagnosed with PFAPA when she was 6 months old. We went through all of the same issues as the rest with our daughter her highest fever was 107.2!! The doctor tols us to stop taking her fevers as it really didn't matter how high it got. My daughter is a patient at the National Institute of Health Peds clinic in Bethesda, MD. She was put on anakinra in July 2011. It has worked well for my daughter when the prednisone did nothing for her.

RHurst said...

My daughter does have a re-occurring fever. Now I am worried she might have this. I will have to schedule and appointment with her ent doctor and get her checked out.

Lauren W said...

This is a great blog. I appreciate reading your story and everyone's comments. My son started getting a recurring fever every 3 weeks 7 months ago. He also has a severe speech delay due to motor planning issues. He is 20 months old and has never said a word. Last month his doctor mentioned PFAPA and referred us to an ENT. We are seeing the ENT next week for a consult. My son's high fever and drooling are back this week so I called his doctor just to update him. Now he wants my son to go to Infectious Disease for a consult. I just want the tonsils and adenoids out! Would it be a bad idea in your opinion to have the T&A before he saw Infectious Disease? His doctor had originally told me ENT would do the tonsillectomy since he has had 6 recorded cases of tonsillitis in 6 months. I just don't want to wait any longer. It takes so long for referrals and appointments to happen. My son goes to speech therapy and he never gets anywhere because he is so often sick. I want him to talk, I want him healthy, I just want my boy to live a normal life.

amy said...

Lauren - If the ENT will do a T&A I would go for it. The benefit of an infectious disease doc is just that he can look at your son as a whole and rule out any other illnesses. Just remember the T&A isn't a guarantee.

Good luck with your son and I hope he is healthy and talking up a storm soon.

Vanessa - Did your daughter have the T&A? I struggled so much with all of the different medications they wanted to try on Ivy. And each one we tried would improve her situation for a few months and then it would get back to fevers every 3 weeks. 107 was our highest as well. I shutter when I come across my "fever journals" and am just amazed when I look at her blood test results.

msimpson said...

My daughter's pediatrician just gave her a preliminary diagnoses of PFAPA. She is 2 and began having fevers on a regular basis 3 weeks after she got a combined vaccine. She has been getting them for 8 months now . . . anywhere from 3 weeks apart to 2 months apart. It breaks my heart to see her suffer. I was wondering if anyone else noticed a correlation between vaccines and the onset of PFAPA.

Lauren W said...

Thanks Amy for writing me back! I was waiting to hear what ENT would say and today he agreed to the "blue plate special": tonsillectomy, adenoidectomy, and PE tubes all at once. I'm so happy! I definitely understand it might not be a cure, but I'm happy to try. We will hold off on seeing Infectious Disease until the surgery is over and see how he does. I'll write back again down the road and let you know how it goes. Thank you so much for your blog.

Mickey, Lauren, Colin, Will's Family Blog said...

Hi Amy, I previously posted about as "Lauren W." Thank you for commenting back. I took your advice and my son saw the ENT last week. He agreed to do T&A and PE tubes at the same time. I'm so happy. His surgery is scheduled for January 9. I just hope he is fever-free that day or they won't do the surgery and he'll have to wait longer. I went back and looked at my calendar noting when fevers started and it's every 19 days most of the time; sometimes up to 21 days. He's due for another episode this Thursday or so and surgery is going to be cutting it close to the next fever episode. Anyway, we'll see what happens. He still has an appointment with Infectious Disease at Children's Hospital next week and we decided we will go there just in case we run into any issues with the fevers coming back after surgery.

I appreciate your blog very much and everyone's comments/advice. I will comment again down the road in a few months after surgery is over and he's hopefully doing better with an update.

Anonymous said...

Thank you for creating this wonderful Blog...want to share my story...my daugther just turned 10 year old in January 1st, she was a very healthy kid until last April she had a constant fever for almost 26 days, ended in the hospital, the fever went away and she got it back every three weeks for 4 months, after so many visits to different doctors her rheumatoid arthritis specialist decided to start on prednisone to diagnose her with PFAPA, I was not happy about her talking steroids, in the mean time I found that also few kids had been cured with PFAPA getting treatment from an upper cervical chiropractor, one of my friends suggest to me to see a physical therapist at this point I had nothing to loose and I went to see her, after her first treatment I noticed her fever last only two days and it was not high as usual (104-105) then the next month it came back for 6 days but didn't exceed more than 102, until they were gone, this treatment is call Myofascial treatment, it's very gentle and works around the Fascia in your body, the inflammation on her body went away, please search and google about Myofascial and PFAPA you'll find a lot of information, now my daughter's rheumatoid arthritis specialist is very interested and want to research more about this, the bottom line is that it worked for her, the inflammation that cause the fever every three weeks exactly (can even predict the time) is gone and that is the most important thing.
Now she is fever free for 4 months and I hope it never came back. The weird part is that kids grow out of this most of the time at the age of 9 and 10 and my daughter got it at the age of nine, she went through so many tests and always negative, doctor always said is a viral infection. Only the two doctor listen to me and were great giving me feedback and willing to diagnose her with PFAPA the rheumatoid arthritis specialist and the infectious specialist, the other doctor's just walk away.
Do not sit and wait for doctor results, do your research and you'll be surprise how much information you can find. If your are interested check the following web sites:
you are not alone!
http://stop-periodic-fever.blogspot.com/2011/05/pfapa-cured-periodic-fever-gone.html
http://www.myofascialrelease.com/fascia_massage/public/default.asp
http://wiki.answers.com/Q/What_are_the_treatments_for_Marshall_Syndrome
http://pedsinreview.aappublications.org/content/30/5/e34.extract

Jeff and Karen said...

My daughter has fevers just about every 4 weeks but for years it was thought to be allergies causing sinus infections. We had her adenoids out with sinus surgery in June and it helped tremendously but then started back in November with fevers. Her fevers are not high usually 99.5 and last for more than a week, usually 10 days or so. She never complains so we don't know if she has headaches or stomachaches - maybe she has them all the time so thinks its normal?The ENT said today that wondered about PFAPA so we're to read the information and get back with him. Does her lack of other symptoms seem like it wouldn't be PFAPA? Her tonsils are now enlarged with a few spots on them but nothing major. Any advice is appreciated.

Anonymous said...

Here's our story...
In October 2006, after months of frequent fevers in our 3yo and being told each time it was a virus because he goes to daycare, a dear friend and I began simply researching the internet for possible causes. We are located in NC and came across a doctor at Duke, Dr. Ross McKinney, whose clinical interests are in "fevers of unknown origin," FUO. I called Dr. McKinney's office and spoke to a PA who suggested I look into PFAPA because the symptoms where similar to what I had described. The good news was IF he was diagnosed with PFAPA, it is benign and that he should grow out of it.

I called the daycare and began putting together a history of this condition in my son. He ran 102-103 degree fevers every 4 weeks lasting approximately 3 days each. I was able to say with certainty it began at least 11 months prior. Armed with that information, we went to meet with Dr. McKinney.

We met with two doctors at Duke who spent lots of time with us. The first doctor, Dr. Cohen, took down details and examined our son for about an hour. Then he discussed his observations (they already had his medical history) with Dr. McKinney and they both came back and talked with us for another 20 or 30 minutes. Dr. McKinney was convinced that our son had PFAPA based upon the history of his fevers and needed no further testing because we had already done the immunity tests the prior December and had CBCs run back in July 2006. He gave us a prescription for Prednisone and instructed us to give him one dose the first day of his next episode and then one more the following morning. The dosage was the same as if he had poison ivy. Dr. McKinney wanted us to do this for the next two episodes to confirm that his diagnosis is correct in our minds (he was already convinced). He was right. The Prednisone stopped the episode within hours of giving it to him. Though the fever went away nearly immediately, it returned 2 weeks later. At that point, we decided only to use Prednisone if the fever was at an inconvenient time because Motrin was fairly effective for our son.

At the time, Dr. McKinney said PFAPA was still a fairly rare syndrome however, they get about 1 new patient per month. Since diagnosis, we have had several people ask us about it because they know others experiencing similar issues.

Our son is now 9. He has not had an episode since July 2008 and we are very grateful. We understand the helpless feeling when your child is suffering and you don't understand what is happening. For us, it was a blessing to receive the diagnosis of PFAPA. There are so many worse conditions that we could have faced. We hope others continue to share their stories and we wish you all the best.

Anonymous said...

Thank you for all the information. My 4 year old daughter was just diagnosed with PFAPA. Her fevers have been twice a month for over a year now. After many trips to the pedi, blood work, cat scan, etc. they have finally diagnosed her.

I have learned alot from reading everyone else's experiences. I am going to push for t/a removal. She is starting school in the fall and I worry about her missing every other week. I will need to consider home schooling if the fevers continue...

Thank you for the information!

Unknown said...

So my update... I posted "amy" on 3/08/2011 10:19:00 AM about my daughter and her PFAPA. Very pleased and relieved that she has now had her T&A removed at the end of March of 2012. A year later she had been in and out of hospitals about once a month. The fevers got worse, on occasion it had peaked at over 104 and were sustained for 3-5 days at 101 or better. So after surgery... So far so good. She pulled through like a champ. Doctors said her tonsils were REALLY bad, a lot larger than expected. Her adenoids were bad as well, even though she didn't have a snoring problem. I am so glad we made the decision to remove. I would do it again in a heartbeat. I am pleased that the doctor was not at all hesitant about removing her tonsils. Her very first appointment with her pediatric ENT, he took one look and got a run down on her other symptoms (sores in her mouth) and said surgery. He explained that he could put her on steroids, or low dose antibiotic if I was more comfortable, but he recommended surgery. Two weeks later surgery complete and she is SOOOO Much better! Praying that I am not speaking too soon, but so far she hasn't had a fever since February!

Anonymous said...

I need help please. My son just got diagnosed with PFAPA I'm not sure what to think at this point and really would like someone to talk to on the phone. My email is patricia06140727@yahoo.com. If you email me I can give you my number. I really need some advice.

Anonymous said...

I see that people are still posting to this and I wanted to add my son's story as well. His story is like sooo many others I have just read here. He is 16 months old right now, and has been experiencing high fevers (103-105 degrees), mouth ulcers, red throat, occasional vomiting every 4th week of each month since he was 8 months old. The episodes generally last 5 days, on a "good month" only 3 days. He has repeatedly tested negative for bacterial infections, and other known "germ" causes have been ruled out at this point. He never has any type of ear infection, cold, cough, etc. He will be tested (bloodwork) for markers for PFAPA when the next episode occurs in approx. 2.5 weeks. We have been able to accurately predict them to a weekend that it will begin - and 2 days prior we know that it will be coming due to a change in his sleep (he is awake almost the entire night with only a few hours of day sleeping for 5 days straight), diet (he barely eats a thing through the entire episode) and behavior (screaming, crying, kicking). His eyes change and habits also change - as noted in your initial story with your daughter. It's a glassy-eyed sad eyes. A totally different child. My son is just learning to use words - so we are unable to know for sure what is going on other than what we can physical see and measure at this point.

I am hoping to get into a pediatric rheumatologist for better support, as we had to specifically ask that he be examined for other causes for the fevers, since we were being brushed off at the pediatricians each time it happened. The last pediatrician we saw, when pressed about another cause, and reminded that his mother (myself) has lupus and we were concerned with an autoimmune type of condition, truly believed us and has pushed for more testing.

I will be seeing my rheumatologist this week, and I hope to discuss my son with him, and I hope to get him into the office to get some help and direction. From what I read here - it really looks like removing his tonsils when appropriate (also a procedure I needed as a child), is the best bet to decreasing/ending the episodes. So an ENT is probably on the list of people to see.

The more I read about other families' stories the more I see that this condition is probably going to last the duration as described in the educational informational Websites I have read, and the sooner I can find the best option to decrease these episodes the better off we will all be.

Thank you all for your posts and stories. It helps to know we are not alone, as it sometimes seems when you are going through repeated episodes.

Anonymous said...

Reading all your experiences I love sons person doctor even more. My 4yr old son has been experiencing episodes for 4 mos but today she sent us off for bloodwork and we have an initial diagnosis of pfapa. Our appetite with a Enter specialist at children's hospital in Boston is the endofAugust. Reading all the posts I will be sure to ask for his tonsils removed. Zachary starts school in the Fall and I donot want him to miss a Ak every month. And as a singleparent I am afraid of losing my job due to needing a sk a month off. Thank you all for sharing you stories.

Bcoby said...

My 10 month old daughter was tentatively diagnosed with PFAPA after 4 months of recurring fevers and sore throat. I kept taking her into the doctor every month because she was just miserable. The doctor would say it was a virus or give me an antibiotic. My daughter had 2 false positive strep tests which upon growing the culture out, proved to be negative. This last month, her fever was 105.7 and her throat was red, swollen and had patches of white pus on it. My heart is broken that she may have to go through this every month for years. My pediatrician referred us to an Infectious Disease specialist that we have an appt. with the end of this month. We pray that they will be willing to recommend that she have her tonsils removed sooner than later. I just can't imagine going through years of her feeling crappy every month. I am a stay at home mother, so I don't have to deal with the stress of taking off work every month, but I do feel guilty that my 4 year old child doesn't get the attention and care she needs when my baby is suffering through one of these episodes.
I have read info. on Marshall's Syndrome which speaks to physical characteristics which are common for the syndrome. I am wondering if any of you with children who have PFAPA see these characteristics in your child?

Patrick said...

To BCOBY:

Patrick here - I have some posts above and had a son who had PFAPA. I read your post and have been through what you are feeling. I have a couple of thoughts for you.

1) Most importantly, it will get better. We didn't figure out what my son had til he was 10 and we discovered PFAPA on our own. You are way ahead of the curve.

2) there are potentially more options out there than even three years ago. In addition to surgery, some people seem to be having a lot of success with Ankira. See the above posts. That being said, our son had surgery and has been healthy as can be since...

3) Keep pushing PFAPA with your doctors until they rule it out (I don't think they will since what you describe screams PFAPA). Chart your daughter's fevers to make your case. I can't stress how important it is to keep this up because when we went through it our pedi hadn't even heard of PFAPA.

4) Just my opinion, but your doctor's approach to the fevers is typical and not helpful in diagnosing the problem. If it is a virus, why prescribe an antibiotic? This was one of the things that drove us crazy. When we didn't use antibotics, the fever would go away in five days and we were told it probably was a virus. When we did use antibiotics (cue the guilt music) the fever would go away in five days, and we were told it was probably and infection that didn't show up on any tests.

5) Find someone in your area who specializes in PFAPA and get an appointment.

Hang in there. My guess is this time next year you will have figured out the issue, and everything will be in the past.

Keep us posted.

Helga Gross said...

My baby girl was diagnosed with PFAPA when she was 9 months old (July 2011). As all of you know, the fevers are high and don't respond to acetaminophen nor motrin. However, whenever my baby girl had her monthly fever, I continuously administered tylenol and motrin, alternating every 3 hours during 4 to 7 days, with the hope of controlling the fever, and yet, her fever would spike resulting in horrible febrile seizures (traumatic for a parent to watch, especially when it's a baby). I had taken her to every possible hospital (Harbor Hospital in Baltimore, University of Maryland, Johns Hopkins, Kennedy Krieger Institute, Mercy Hospital and Nation's Children's Hospital in Washington DC) as well as specialist such as hematologist, rheumatologist, neuro pediatrician, immunologist, allergist, infections disease specialist, and finally she was diganosed with PFAPA by Dr. Barbara Jantausch, an infections disease specialist at the
Nation's Children's hospital in DC, who does research with the NIH and is very well acquainted with the syndrome. At first she explained the 3 most common types of treatments:
1. steroids (prednisone) - which she did not recommend, as although the fever would end quickly, the frequency would most likely increase, plus, it's steroids, which are never good for you, let alone for a baby.
2 - tonsillectomy - she said that it was the most efficient form of treatment with 80-90% chance of cure, but knowing that my little one was just a baby that wasn't even 1 year old yet, her body/immune system was still developing, that was out of the question, definitely not an option at that moment for me.
3 - Cimetidine - she explained that cimetidine was an anti-acid commonly used to treat ulcer that was all about 1/3rds to treat PFAPA, that is, 1/3 chance that would cure completely, 1/3 chance that would decrease the number of days/frequency, and 1/3 chance that it would not work at all, but this was the form of treatment that she suggested me to start with.

Helga Gross said...

My baby girl had 5 terrible febrile seizures due to the fever spiking to 105-106 degrees. The last time she had a seizure (last year - April of 2012)we were on vacation in Brazil, where I'm originally from, but I live in the US). Since she had the fever while we were there, I took my little one to my pediatrician (from when I was a kid 3 decades ago), Dr. Avelino Neta Ramos. He ordered some lab work and while my baby girl was having her blood drawn, Dr. Avelino did some research on PFAPA and learned that the syndrome does not respond to tylenol nor motrin, so once he got the results back, and as always, the results are always normal with the exception of the white blood count which is always high during the episodes, he prescribed me an over the counter medicine called Dipirona Sodica.
IT WAS LIKE A MIRACLE HAD JUST HAPPENED!!! As soon as she took the medicine, her fever started to drop, I couldn't believe my eyes, I've cried with joy. The medicine is used for fever and pain, just like acetaminophen and motrin, however, besides it being more natural with less to no side effects, it can be used for a lot of other treatments. Also, I would administer every 6 hours, instead of every 3 hours (from alternating tylenol and advil, which is so bad for the liver/stomach), and on top of that, the fever that used to last an average of 5 - 7 days, reduced to 1 - 3 days. Hence, when I returned to the US, I brought a lot of the Dipirona Sodica with me.

At that time, I had a next-door neighbor who happened to be a pediatrician as well as a clinical doctor. When I returned from Brazil and talked to her about the Dipirona Sodica, she said she was vey well acquainted with that medicine since she had gone to Haiti as part of the Doctors without Borders' team, to assist when the earthquake happened. She said that there were a lot of injured people that needed care, and Brazil had donated a large supply of Dipirona Sodica, which was used to treat just about everything, and according to her, worked wonders. I've asked her why that medicine was not FDA approved in the US, and she said that if Dipirona was approved in the US, it would destroy Tylenol and Advil, which are big brand names, with big companies that pay big bucks to the FDA. I can't prove that, but it makes perfect sense. Dipirona can be found in many countries in Europe, South America, Africa, but not in the US, such a shame.

As a parent, I had to share about this medicine, as I grew up taking it and couldn't imagine that it would give me sanity/relief after so many sleepless nights taking care of my baby. Now when she has her fevers, at least, I can be rest assured that her fever won't go any higher than 101-102 - the average is 100-101 and she won't ever have another seizure. She can sleep better and doesn't have to be woken up every 3 hours to take medicine. From the bottom of my heart, this medicine really made a difference in our lives. For any parent who is looking for an alternative, this is one that works, at least to really control the fever and reduce the frequency.

Anyway, I've also continued with the cimetidine treatment, which seems to be working, slowly. About 2 months ago I took my little one to see Dr. Jantausch, and while we were talking she said that new researches presented different results for the tonsillectomy, saying that it is not so effective as it was thought to be, as more and more people who had the tonsils removed are experience and thought were cured, ended up experiencing the episodes again, on a monthly basis.

Should anybody have any questions, please let me know, and I'll gladly share information.

Kara said...

We had the same problem that our daughter w PFAPA threw up the prednisone. We just got new dissolvable tablets instead of liquid and those work!

Kara said...

We had the same problem that our daughter w PFAPA threw up the prednisone. We just got new dissolvable tablets instead of liquid and those work!

Elizabeth said...

My daughter was diagnosed when she was 3 with PFAPA. She is now 11. We have been up and down on the roller coaster of fevers. Some years she was fever free. We have never used any steroids or meds except tylenol and motrin. I have always been afraid to give her the steroids. Just recently in the spring, she had recurring fevers every month. We decided she needed a tonsillectomy in July 2014. We did not have her adenoids removed though. not sure if that was a good or bad decision. She has been fever free since July. She did get a fever a day ago and it has been between 99 and 100. We are hoping this fever will end and no more will return. Any thoughts on other children who had tonsils removed and if there fevers returned?

Amy Sanford said...

Elizabeth - it has now been several years since Ivy's surgery. She did do the tonsils and the adenoids. We have not had a high fever since the surgery. We do still have "episodes" where she has other symptoms and may have a low grade fever (rarely over 100). We are so grateful it has worked.